POS0778 METASTATIC ANGIOSARCOMA MASQUERADING AS SYSTEMIC INFLAMMATORY DISEASE WITH REFRACTORY PERICARDITIS
Authors: K. Williamson
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Rheumatologists are frequently asked to evaluate patients with acute pericarditis. The majority of cases are idiopathic with systemic autoimmune disease underlying maybe 7% or less of cases in developed countries.<sup>[1-2]</sup> It is critical to recognize the presence of systemic symptoms or poor prognostic pericarditis features, such as tamponade physiology or poor response to non-steroidal anti-inflammatories, that raise concern for a malignant etiology of pericarditis.<sup>[1-2]</sup> <h3>Objectives</h3> A case report of a patient presenting with refractory pericarditis caused by metastatic angiosarcoma. <h3>Methods</h3> A 46 year old male presented for evaluation of unresolving pericarditis. Five months prior, he had developed abrupt-onset pericarditic chest pain with associated dyspnea. Initial ED evaluation was notable for normal troponins and non-specific T wave abnormality on ECG. CT chest angiogram revealed a trace pericardial effusion and isolated right axillary lympadenopathy. The patient declined further evaluation. He presented a week later with persistent pericarditic pain. Echocardiogram showed enlargement of the pericardial effusion with possible early tamponade. Respiratory viral testing was negative. C-reactive protein (CRP) was elevated to 20.83mg/dL (Ref ≤0.5mg/dL) and ferritin to 2344.4ng/mL (Ref 21.8-274.7ng/mL). Soluble IL-2 receptor and blood counts were normal. Rheumatoid factor was weakly positive with ANA, anti-DNA, anti-CCP, and ANCA returning negative. Core needle biopsy of an axillary lymph node was negative for malignancy. Pericardiocentesis was deferred due to patient preference and stability. He was started on colchicine for autoimmune versus idiopathic pericarditis without significant improvement. He was transitioned to prednisone with improvement in his chest pain, effusion, and CRP; dyspnea and hyperferritinemia persisted. Attempts to taper prednisone failed due to flaring of pericarditis. The patient was referred to our center for evaluation of refractory pericarditis and possible systemic inflammatory disease. No signs or symptoms of RA or CTD were present. The patient endorsed recent night sweats, 11 kg of weight loss, and new bone pain. Full body PET/CT was pursued and demonstrated two FDG avid masses adjacent to the right side of the heart along with multiple lytic bone lesions of the spine and pelvis. <h3>Results</h3> Cardiac CT confirmed malignant-appearing, centrally necrotic mass along the right side of the heart extending to involve the pericardium. Iliac bone biopsy confirmed a diagnosis of metastatic angiosarcoma. He was not a candidate for cardiac tumor resection and was referred to oncology for palliative chemotherapy. <h3>Conclusion</h3> Primary malignant cardiac tumors are a rare cause of pericarditis. Cardiac angiosarcomas are the most common type of primary malignant cardiac tumor. They most commonly arise from the right atrium or pericardium and may present with pericarditis-like symptoms.<sup>[3]</sup> <h3>References</h3> [1]Gouriet F, Levy PY, Casalta JP, Zandotti C, Collart F, Lepidi H, Cautela J, Bonnet JL, Thuny F, Habib G, Raoult D. Etiology of Pericarditis in a Prospective Cohort of 1162 Cases. Am J Med. 2015 Jul;128(7):784.e1-8. doi: 10.1016/j.amjmed.2015.01.040. Epub 2015 Mar 10. PMID: 25770033. [2]Imazio M, Cecchi E, Demichelis B, Ierna S, Demarie D, Ghisio A, Pomari F, Coda L, Belli R, Trinchero R. Indicators of poor prognosis of acute pericarditis. Circulation. 2007 May 29;115(21):2739-44. doi: 10.1161/CIRCULATIONAHA.106.662114. Epub 2007 May 14. PMID: 17502574. [3]Joseph AL, Wilklow FE, Olivier JJ, Joseph GJ, Glancy DL. Angiosarcoma of the Heart Presenting as Subacute Pericarditis. Am J Cardiol. 2017 Mar 15;119(6):941-943. doi: 10.1016/j.amjcard.2016.11.049. Epub 2016 Dec 18. PMID: 28043397. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.